baÁt tÖÔng hÔÏp nhÓ thaÁt vaØ thaÁt ÑaÏi ÑoÄng...
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BABAÁÁT TT TÖÖÔNG HÔÔNG HÔÏÏP NHP NHÓÓ THATHAÁÁT VAT VAØØTHATHAÁÁT T ÑÑAAÏÏI I ÑÑOOÄÄNG MANG MAÏÏCHCH
Ths.BSThs.BS PHAN HOAPHAN HOAØØNG THUNG THUÛÛY TIEÂNY TIEÂN
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Case Case laâmlaâm sasaøøngng :1 :1 sasaûûnn phuphuïï 33 33 tuotuoååii ,,cocoùù thaithai 33 33 tuatuaàànn 5 5 ngangaøøyy ñöñöôôïïcc chuyechuyeåånn ññeeáánntrungtrung taâmtaâm vvìì phaphaùùtt hiehieäänn nhòpnhòp timtim thaithai chachaäämm khikhi khakhaùùmm thaithai ññònhònh kykyøø
-4buoàng :+daïng thaát phaûi thieåu saûn ôû treân nhó traùi vaødaïng thaát traùi ôû treân nhó phaûi+ÑMP noái vôùi thaát traùi vaø daõn nheï(8,4mm)+ÑMC leân heïp (5,5 mm) vaø noái vôùi thaát phaûi-Doppler:+Hôû van 3laù naëng (4,6m/sec)
+Gd qua van 2 laù, ÑMC, ÑMP bìnhthöôøng
Chaån ñoaùn :Baát töông hôïp nhó thaát vaø thaát ñaïi ñoäng maïch keøm thieåu saûn thaát phaûi ,ÑMC leân ,Bloc nhó thaát hoaøn toaøn baåm sinh
SA tim thai:
-Situs solitus
-Tyû leä chu vi tim thai /loàng ngöïc =0,65
-bloc nhó thaát hoaøn toaøn /M mode :nhòp nhó155l/ph, nhòp thaát 53l/ph
Sinh hoùa thai: phuø hôïp tuoåi thai
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ÑÑÒNH NGHÒNH NGHÓÓAA
--ThaThaáátt dadaïïngng thathaáátt tratraùùii nanaèèmm beânbeân phaphaûûii vavaøø nonoááii vôvôùùii ññooäängng mamaïïchch PhoPhoååiiThaThaáátt dadaïïngng thathaáátt phaphaûûii nanaèèmm beânbeân tratraùùii vavaøø nonoááii vôvôùùii ññooäängng mamaïïchch ChuChuûû
--NhNhóó phaphaûûii nonoááii vôvôùùii thathaáátt tratraùùii qua van 2laqua van 2laùùNhNhóó tratraùùii nonoááii vôvôùùii thathaáátt phaphaûûii qua van 3 qua van 3 lalaùù
•-
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HHììnhnh aaûûnhnh sieâusieâu aâmaâmtataààngng nhnhóóTMP TMP ññooåå veveàà nhnhóó tratraùùiiHôHôïïpp llööuTMganuTMgan, , TmchuTmchuûû ddööôôùùii ññooåå veveàà nhnhóó PhaPhaûûiiNhNhóó TraTraùùii cocoùù van van VieussenVieussen
taàng thaátthaát Phaûi
van 3laù gaàn moûm tim hôn van 2laùCoât cô gaén vaøo vaùch lieân thaátModerator band naèm gaàn moûm
Thaát Traùithaønh meàm maïi, coät cô van 2laù
khoâng gaén vaøo vaùch lieân thaát
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Moderator bandModerator band
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4 4 buobuoààngng
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ÑöÑöôôøøngng rara cucuûûaa thathaáátt ÑÑMPhoMPhoååii
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ÑöÑöôôøøngng rara cucuûûaa thathaáátt: : ÑÑMC, MC, ÑÑMPMP
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VideoVideo
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VideoVideo
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DopplerDoppler
-Phaùt hieän thoâng lieân thaát lôùn -Baát thöôøng van nhó thaát (hôû van 3 laù) -Heïp van ÑMP
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TyTyûû leleää mamaéécc--<1% <1% bebeäänhnh timtim babaååmm sinhsinh--Nam Nam ththööôôøøngng gagaëëpp hônhôn nnööõõ
Baát thöôøng lieân quan -Thoâng lieân thaát (50%)
-Heïp van hoaëc döôùi van ÑMP (45%)-Hôû van 3 laù ,Ebstein-NST : Monosomy 22q
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,,
Case ls (tt)
-Choïc oái :keát quaû NST bình thöôøng-Kieåm tra SAÂ haøng tuaàn ñeå phaùt hieän daáu hieäutaêng suy tim vaø leân keá hoaïch döï sanh quanh tuaàn38- Sanh thöôøng 1beù trai naëng 2565g lucù 37tuaàn 3 ngaøyÑaùnh giaù sau sanh : gioáng tröôùc sanh(BTHNTTÑÑM,thieåu saûn thaát phaûi, ÑMC leân ,hôûvan 3laù naëng ) +van ÑMC 1 maûnh, heïp naëngBeù khoù PT, ñöôïc ñieàu trò hoài söùc noäi khoa tích cöïc
•Baøn luaän
•-Tieân löôïng tröôùc sanh :nhòp tim chaäm, baát thöôøng caáu truùc tim,aûnh höôûng huyeát ñoäng cuûa beänh van 3 laù
•-Khoâng loå van ÑMC cô naêng khoâng ñöôïc ñoaùn tröôùc sanh vì vaãn coùdoøng maùu qua van ÑMC heïp (maøu, doppler)vaø nguyeân nhaân coù theå dosöï thay ñoåi huyeát ñoäng khi sinh
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TieânTieân llööôôïïngng--NhNhööõngõng yeyeááuu totoáá ththööôôøøngng gaâygaây ttöûöû vongvong ::suysuy thathaáátphatphaûûii, , hôhôûû van 3 van 3 lalaùù,va,vaøø bloc bloc NT NT hoahoaøønn toatoaøønn-- BTHNTTBTHNTTÑÑÑÑM M kekeøømm TLT: TLT: tientienáá trietrieåånn ttöïöï nhieânnhieân ththööôôøøngng chachaäämm hônhôn TLT TLT cucuøøngng côõcôõ--BTHNTTBTHNTTÑÑÑÑM M kekeøømm TLTphoTLTphoááii hôhôïïpp heheïïpp van van ÑÑMP :MP :tietieáánn trietrieåånn giogioáángng
T4F T4F
Ñieàu tròBTHNTTÑÑM ñôn thuaàn : khoâng caàn söûa chöõaCan thieäp ngoaïiï khoa: söûa nhöõng toån thöông phoái hôïp quyeát ñònh trieäu chöùng laâm saøng ( thoâng lieân thaát, heïp van ÑMP )
Rastelli procedure
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TOTOÙÙM TAM TAÉÉTT--XaXaùùcc ññònhònh nhnhóó phaphaûûii, , nhnhóó tratraùùi(vani(van vieussenvieussen))--XaXaùùcc ññònhònh thathaáátphatphaûûii, , thathaáátt tratraùùii ((ssöïöï gagaéénn van van nhnhóó thathaáátt, , --Moderator Moderator band)band)--ÑÑMC ,MC ,ÑÑMP(chiaMP(chia ññoâioâi sôsôùùmm))--KhoângKhoâng cocoùù dadaááuu babaéétt checheùùoo 2 2 ññaaïïii ÑÑMM--BeBeäänhnh kekeøømm (CIV, (CIV, EbsteinEbstein ))--Hay Hay gaâygaây rara biebieáánn chchöùöùngng roroááii loaloaïïnn nhòpnhòp ((NhòpNhòp chachaäämm , Bloc NT, Bloc NT……))